David G. Pugh

Skeletal Lesions in Neurofibromatosis

Neurofibromatosis (Recklinghausens disease) is a congenital and familial disease presenting abnormalities of the skin, nervous system, bones, and soft tissues. A classic case demonstrates (1) multiple soft, elevated cutaneous tumors (fibroma molluscum), (2) cutaneous pigmentation (cafe au lait spots), and (3) neurofibromas of peripheral nerves, frequently palpable in the subcutaneous tissue. In addition to this “cardinal triad” of diagnostic findings may be seen elephantoid soft-tissue masses on the body and fan-like enlargements of peripheral nerves called “plexiform neuromas.” Incomplete varieties of the disease (formes frustes), showing only cutaneous pigmentation, also occur; these cases frequently progress to the complete picture later in life. In the last few decades, the variety of skeletal defects associated with neurofibromatosis has received increasing attention. These are of particular interestto the radiologist, since certain types are characteristic of the disease, while others are most sugg...

Aneurysmal Bone Cysts

In a comprehensive review of the records of more than 2,000 primary bone lesions encountered at operation at the Mayo Clinic in the period 1905–52, 26 aneurysmal bone cysts were found. The lesion first was recognized by Jaffe and Lichtenstein (1), this being one of the many contributions they have made in the field of bone pathology. Our investigation reaffirms their belief that aneurysmal bone cyst is a distinct clinical and pathologic entity. The specific term “aneurysmal bone cyst” does not appear in the literature until 1942, when Jaffe and Lichtenstein stated for the first time that it was probably a distinct entity and described the findings in 2 cases. In many papers prior to this (2–7), especially those concerning giant-cell tumors and their “variants,” more or less complete descriptions of the lesion are encountered under a variety of names. Ewing (8), in 1940, employed the term “aneurysmal giant-cell tumor” for the condition, which he considered to be a benign variant of a giant-cell tumor takin...

Psoriatic Arthritis: A Roentgenologic Study

The concept that psoriatic arthritis is a distinct clinical entity has been endorsed by many, questioned by some, and rejected by others. A considerable number of authors are of the opinion that changes in the joints of patients with psoriasis are indistinguishable from those of rheumatoid arthritis and that unusual cases are atypical forms of the latter disease. On the other hand, psoriatic arthritis has been distinguished clinically from ordinary rheumatoid arthritis in many respects, particularly in the involvement of the distal interphalangeal joints together with concurrent activity of arthritis and psoriasis of the nails and skin, and the usually good response to therapy in early cases. The concept that psoriatic arthritis is a distinct clinical entity has been endorsed by many, questioned by some, and rejected by others. A considerable number of authors are of the opinion that changes in the joints of patients with psoriasis are indistinguishable from those of rheumatoid arthritis and that unusual ...

Massive Osteolysis and Angiomatosis

Massive osteolysis (phantom bone, spontaneous absorption of bone, or disappearing bone disease) is a bizarre entity characterized pathologically by angiomatosis. Its etiology is unknown, the clinical progression and prognosis are unpredictable, and treatment is uncertain. Absorption, initially localized to one bone, often extends to involve those adjacent to such a degree that clinically the ultimate impression can hardly be considered anything less than “malignant.” The associated angiomas, however, are histologically benign and no pathologically malignant tissue is present. Deformity and disability may be extensive; widespread involvement of ribs and vertebrae may cause death. Since the first case of spontaneous absorption of bone recorded by Jackson (7, 22) in 1838, at least 38 additional cases have been documented. Gorham and Stout (9, 10), in a review of 24 of these, emphasized the hemangiomatous nature of the bone in the zones of osteolysis. Three years later, Johnson and McClure (17) reviewed 8 add...

Roentgenologic Changes of the Skeletal System in Cushing's Syndrome

Although many articles have been written about the clinical features, pathology, and pathologic physiology of Cushings syndrome, only two reports have dealt primarily with the roentgenographic aspects (1, 2). These and other studies have indicated that the most characteristic skeletal roentgenographic findings are osteoporosis and attendant fractures, without features peculiar to Cushings syndrome. The present report was stimulated by an impression obtained by one of the authors (Pugh) that the spinal osteoporosis in this syndrome frequently is accompanied by an unusual condensation of the margins of some of the collapsed vertebral bodies of a degree seldom seen in other forms of osteoporosis. From a physiologic standpoint, the osteoporosis of Cushings syndrome and most of its other clinical and laboratory features (abnormal distribution of fat, muscular wasting and weakness, hypertension, amenorrhea, impotence, cutaneous atrophy and striation, easy bruising, hirsutism, acne, lymphopenia, eosinopenia, ...

Surgical Treatment of Coarctation of the Aorta in a Ten Week Old Infant: Report of a Case

Although most patients with coarctation of the aorta survive to early adult life, it has been adequately demonstrated that some patients die in infancy and early childhood as a result of their coarctation. In selected cases, then, operation is advisable at a very early age. Cardiac failure in a 10 week old infant necessitated operation at this age in the case reported.

Left ventricular mechanical efficiency in man with heart disease.

Thirty-eight adults with valvular and/or myocardial disease had heart catheterization with coronary blood flow and myocardial 02 consumption (MVO2) per 100 g measured by the nitrous oxide washout technique. Quantitative biplane angiocardiography was performed to assess left ventricular volume, mass, ejection fraction and work. Left ventricular efficiency was calculated from work, MVO2/100 g and mass. Efficiency ranged from 4 to 40% and was normal in some patients with severe ventricular pressure-volume work overloads. Total left ventricular MVO2 ranged up to 461 ml/min. Neither total MVO2 nor MVO2/100 g was significantly related to ventricular work, ejection fraction, or tension-time index. These data suggest 1) a relationship between left ventricular efficiency and myocardial function in chronic valvular or myocardial disease, 2) that efficiency may be normal in hypertrophied ventricles, and 3) that chronic increases in resting ventricular metabolic requirements are met by hypertrophy rather than by increased MVO2/100 g.

Scleroderma of the small intestine.

Involvement of the small intestine in scleroderma appears to be more common than was formerly supposed. The cause of the disease is obscure, and the clinical states associated with it range from spruelike syndromes to conditions resembling mechanical intestinal obstruction. In a series of 11 patients, the most prominent gastrointestinal complaint was vomiting. The most characteristic roentgenographic finding was persistent dilatation of the intestinal loops after passage of barium.

Giant-Cell Tumor of Bone'

THE PRESENCE of multinucleated giant cells in a variety of bone lesions has led to inclusion of certain neoplastic, inflammatory, developmental, and metabolic disorders of bone under the diagnosis of giant-cell tumor or giant-cell tumor variant. In 1940, Jaffe, Lichtenstein, and Portis (1) offered a specific definition of giant-cell tumor which has gained wide, though not universal, acceptance. They considered it a distinctive neoplasm apparently arising in the nonosteoblastic connective tissue, composed of a vascularized network of spindle-shaped or ovoid stromal cells interspersed with multinuclear giant cells (which they regarded as syncytial stromal cells). In subsequent publications Jaffe and Lichtenstein (2–6) identified as distinct clinical and pathologic entities many of the lesions formerly classed as giant-cell tumor variants. Accurate identification of lesions in this category is of considerable practical importance. Most of the socalled giant-cell tumor variants are fundamentally benign lesion...

Localized Destructive Lesions in Rheumatoid Spondylitis

The ordinary roentgenologic manifestations of rheumatoid spondylitis have been well described (1–4). Clinicians and roentgenologists are especially interested in recognizing the early phases of the disease, when treatment is often efficacious; little improvement can ordinarily be effected when the condition is far advanced. For that reason, little has been written concerning the late stages of the disease, except for descriptions of operative procedures that have been used to correct extensive deformity. The so-called bamboo spine, in which the spinal column is completely ankylosed as the result of ossification of the vertebral ligaments, is easily recognized. It is usually stated that in these cases the vertebral bodies are osteoporotic and the intervertebral disks are narrowed but that neither structure is directly involved or affected by the disease (5). This investigation was initiated by the observation that certain patients show striking destructive changes involving the intervertebral disks and adj...